British Journal of Dermatology (1992) 126. 523-529.
Correspondence Crohn's disease in association with hidradenitis suppurativa SiK. We were interested to read the report by Ostlere. et al.' describing three cases of tiidradenitis supptirativa (HS) in Crohn's disease. We wish to add further support to this association by reporting tvi'o similar cases. We are also aware of iiii earlier report ol' Crohn's disease occurring in a 1 Z-year(»id Black tnan in association with a seroncgative arthropathy itnd the 'follicular occlusion triad' of severe acne. HS itnd dissecting cellulKis ofthe scalp.-^ No details were provided ofthe distribution ofthe HS. but the Crohn's disease affected both the large and small bowel. The tirst case involved an obese lamaictin male who developed nujltiple ischiorectal and groin abscesses with sinus formation at tho ttge of I y years. This was followed (i months later by similar involvement of both axillae. There was no preceding history of acne and he denied any howel symptoms. Iniliul skin biopsies from the perianal area were reported as showing abscess formation with chronic intlammatioii. A clinical diagnosis of perineal Crohn's disease was suspected and during the next year, despite three incision and drainage procedures he continued to develop new abscesses, Investigation of his large bowel, by coloiioscopy and biopsy, showed numerous epithelioid granulomata in the lamina propria with extension into the submucosa. consistent with Crohn's disease. Treatment with sulphasalazine. metronidazole. prednisolone and azathioprine was unhelpful and a hemicok'Ctomy and ileoslomy were therefore performed. Despite this procedure no improvement was seen in the perineal suppuration. In view of thf poor response ofthe cutaneous lesions to treatment ofthe Crohn's disease further skin biopsies were obtained and reviewed. These subsequent biopsies were compatible with chronic suppuration due to HS. Kigliteen months after presentation he developed a suhacute demyelinating peripheral neuropathy possibly related to the chronic suppuration or Crohn's disease. His recovery was complicated by the development of sacral osteomyelitis. The HS has been controlled by surgical excision and split-skin grafts. Case 2 was a 29-year-old female who presented with a f>year history of recurrent painful abscesses predominantly of the axillae, but also occasionally involving the groins. There was no premenstrual flare ofthe lesions. A clinical diagnosis of HS was made and she was treated witb minocyline 100 mg twice daily and topical erythromycin, with initial success. One year after presentation she developed symptomatic Crohn's disea.se with exiensive involvement ofthe large bowel, and a hemicolectoiny was required. She continued to develop abscesses and sinuses in the axillae and a skin biopsy was consistent with HS. thus excluding cutaneous Crohn's disease. Subsequent control of the HS was achieved with combined cyproterone acetate and ethinylm^stradiol. These cases support the association between HS and Crohn's disease. However two important diflerences were seen in our cases compared with those of Ostlere et a/.' Firstly, unlike the previous cases, the HS in our patients preceded the diagnosis of
Crohn's disease (by 1 and f> years). Secondly, axillary involvement was a prominent feature in both of our cases. In our cases, and those previously reported, the Crohn's disease has been severe and has mainly involved the large bowel. All required a colectomy for their gastrointestinal disease. When extensive HS affects the perineal region, as in the first case, it may be difficult to distinguish between HS and Crohn's disease either by clinical or histological criteria. The presence of HS may therefore go unrecognized. However, additional involvement of the axilla favours a diagnosis of HS as metastatic Crohn's disease of the skin usually affects the lower extremities' and has not been reported at this site. The role of local factors in HS has been emphasized'' although the association with Crohn's disease suggests that HS may be a manifestation of underlying disease. Further studies are needed to test the specificity of this association. IX-parlment of Dermatology. Hospital.
N.P.BURROWS R.RtissELL JONES
Road. Soulhall. Middlesex UBl WW. U.K. References 1 Ostlere 1^, Langtry (AA. Mortimer PS, Statjghtoii Rl'll. Hidradenitis suppurativa in Crohn's disease. Or / Dermatol 199 1; 125: i84-f). 2 Kllis Bl, Shier CK, I^isen ]] et ai Acne-associated spondylarthrnpathy: radiographic features. Radiototty 1987; 162: '541-5. i Shum D1\ tJLienlliifr L. Mctastalic Crohn's disease. Arch Dermatol I'^yO; 126: f>45-8. 4 Dvorak VC. Koot RK. Macdregor RR. Host-defence mechanisms in hidradenitis suppurativa. Arch Dermatol 1977: 111: 450-J.
Norwegian scfibies and herpes simplex in a patient with chronic lymphatic leukaemia and hypogammaglobulinaemia SiK. Norwegian scabies is a crusted non-itchy rash caused hy massive infestation with the mite Sarcoptes .^cabiei. This was originally described in patients with Down's syndrome' and leprosy- and more recently in patients who are immunosuppressed by leukaemia. * drugs^ or HIV infection.'^ We report a case of focal crusted scabies occurring on the face of a patient with chronic lymphocytic leukaemia (CLJJ and concurrent herpes simplex. A 67-year-oId man with a lO-year history of CU.. treated with intermittent chlorambuci! and prednisolone, presented with a crusted lesion on his upper lip at the site of a recent herpes simplex infection. He had suffered from recurrent facial herpes for J years and had had an attack of itchy scabies 2 years previously. At the time of presentation he was taking oral acylovir and prednisolone each day. He had been receiving regular infusions of gammagiobulin as he suffered from recurrent chest infections and was hypogammaglobulinaemic. S23
524
CORRESPONDENCE
British Journal of Dermatohgy {1992) 126.
References 1 Wilkinson DS. Norwegian (crusted) scabies, lir I Dermatol 31. 2 Atiderson NP. Stout M. Norwegian scabies. Arch Dcrtmttol Sf n4(): 42: 499-=i()(). i Suzumiya ]. Sumiyoshi .\. Kuroiti V. inmic S. Crusted (Norwegian) scabies wiih adult T-cell leukaemia. Arch Dermatol I98S: ! 2 l : 90i-4. 4. Faterson Wl). Allen BR. Uevcridgt- CW. Norwegian scabies during immunosupprt'ssive therapy. Br \kd I 1973; 4: i l 1-2. 5 Glover A. Young L. Goltz AW. Norwegian scabies in inquired immunodcRciency syndrome: report of ii cast: rt'.sulting in death from associated sep.sis (letlt-rl. / Am Acad Di'rmalol 1987:16; 39()-9.
Scarring alopecia and psoriasis
l-'igure 1. Ik'iivily trusU'd lt'.-,iuns u\ Ninwt'gidii sciibies iiilestiiliiJii at
(he site ol'recenl herpes simplex iiifcilion.
On examination he had gent'ralized lymphadenopathy and heputosplenoincgaly. The lesion on his face was initially vesicular and typical olherpes simplex. A thick crust developed during the next week, spreading out from the nasolabial Ibid and upper lip (Figure 1), Scrapings from the lesion contained large numbers of scabies mites. The lesion resolved completely after 2 months of topical treatment with gamma benzene hexachloride. Norwegian scabies normally affects the hands, feet and trunk and produces a widespread rash suggestive of psoriasis or contact dermatitis. This patient was unusual in that only the face was aftected and that the infestation was so localized. This is the first description of scabies occurring at the same site and at the same time as reactivation of herpes simplex. This case illustrates that impairment of host of defence allows not only infection with atypical organisms but also atypical presentations of more familiar infections. It is important that scabies infestation is included in the differential diagnosis of any immunocompromized patient with a crusted rash, even if other infections are diagnosed at the same site. St Richard's tlospilal Cbichester, W. Sussex. U.K.
C.J.TlBBS D.J.WlLCOX
SIR. Until recently it was believed that hair loss was not a symptom of scalp psoriasis.' It is now well established that psoriatic alopecia is not restricted to patients with erythrodermic and generalized pustular psoriasis, but may al.so occur in patients with chronic plaque p.soriasis.-^ Recently we have shown that hair plucks taken from psoriatic plaques on the scalp have a significantly higher telogen count than normal scalp.' Scarring alopecia in psoriasis has been documented infrequently,^ ^ and we report a further case of this association. The clinical picture and histopathological appearances suggested a single disease entity rather than a coincidental occurrence of psoriasis and scarring alopecia of unknown cause. An 18-year-old woman, suffering from Turners syndrome and epilepsy, presented with a fi-year history of hair loss. She gave no previous history of psoriasis, and there was no family history of psoriasis. She was receiving regular treatment with carbamazepine 200 mg twice daily, and a coinbination ofO-1 5 mg desogestroi and 0 0 2 mg ethinyleslradiol. Her scalp had been treated with topical coal tar and corticosteroid preparations. Examination revealed a large, well demarcated area of scarring alopecia over the occipital and retro-auricular regions of the scalp. Within this area there were no tufts of hair or pustules. Al the margins of the area there was silvery scaling of the scalp which surrounded hair shafts, producing the clinical picture of tinea amiantacea. Skin swabs taken from the border of the area grew Staphtikuocctis utireus; cultures for fungi were negative. Histology of biopsies taken from the scaly margin revealed abnormalities al various levels (Fig. la, and h). The interfollicular epidermis showed hyperkeratosis with orthokeratotic and parakeratotic areas, acanthosis with thinning of the suprapapillary plate, and deep rete ridges. Micn)abscesses of Munro and micropustulesof Kogoj complelett Ihc epidermal picture. The dermis ctintained a mixed intiammatory inliltrate of mononuclear cells and polymorphonuclear leucocytes. The number of hair follicles was decreased. Some damaged hair follicles were observed, partly surrounded and penetrated by leucocytes. No hydropic degeneration was ohserved. The patient was treated with 10% salicylic acid ointment, desoxymethasone cream and betamethasone 1 7-vaIerate scalp application. This treatment resulted in a reduction of the scaling.
Correspondence Crohn's disease in association with hidradenitis suppurativa SiK. We were interested to read the report by Ostlere. et al.' describing three cases of tiidradenitis supptirativa (HS) in Crohn's disease. We wish to add further support to this association by reporting tvi'o similar cases. We are also aware of iiii earlier report ol' Crohn's disease occurring in a 1 Z-year(»id Black tnan in association with a seroncgative arthropathy itnd the 'follicular occlusion triad' of severe acne. HS itnd dissecting cellulKis ofthe scalp.-^ No details were provided ofthe distribution ofthe HS. but the Crohn's disease affected both the large and small bowel. The tirst case involved an obese lamaictin male who developed nujltiple ischiorectal and groin abscesses with sinus formation at tho ttge of I y years. This was followed (i months later by similar involvement of both axillae. There was no preceding history of acne and he denied any howel symptoms. Iniliul skin biopsies from the perianal area were reported as showing abscess formation with chronic intlammatioii. A clinical diagnosis of perineal Crohn's disease was suspected and during the next year, despite three incision and drainage procedures he continued to develop new abscesses, Investigation of his large bowel, by coloiioscopy and biopsy, showed numerous epithelioid granulomata in the lamina propria with extension into the submucosa. consistent with Crohn's disease. Treatment with sulphasalazine. metronidazole. prednisolone and azathioprine was unhelpful and a hemicok'Ctomy and ileoslomy were therefore performed. Despite this procedure no improvement was seen in the perineal suppuration. In view of thf poor response ofthe cutaneous lesions to treatment ofthe Crohn's disease further skin biopsies were obtained and reviewed. These subsequent biopsies were compatible with chronic suppuration due to HS. Kigliteen months after presentation he developed a suhacute demyelinating peripheral neuropathy possibly related to the chronic suppuration or Crohn's disease. His recovery was complicated by the development of sacral osteomyelitis. The HS has been controlled by surgical excision and split-skin grafts. Case 2 was a 29-year-old female who presented with a f>year history of recurrent painful abscesses predominantly of the axillae, but also occasionally involving the groins. There was no premenstrual flare ofthe lesions. A clinical diagnosis of HS was made and she was treated witb minocyline 100 mg twice daily and topical erythromycin, with initial success. One year after presentation she developed symptomatic Crohn's disea.se with exiensive involvement ofthe large bowel, and a hemicolectoiny was required. She continued to develop abscesses and sinuses in the axillae and a skin biopsy was consistent with HS. thus excluding cutaneous Crohn's disease. Subsequent control of the HS was achieved with combined cyproterone acetate and ethinylm^stradiol. These cases support the association between HS and Crohn's disease. However two important diflerences were seen in our cases compared with those of Ostlere et a/.' Firstly, unlike the previous cases, the HS in our patients preceded the diagnosis of
Crohn's disease (by 1 and f> years). Secondly, axillary involvement was a prominent feature in both of our cases. In our cases, and those previously reported, the Crohn's disease has been severe and has mainly involved the large bowel. All required a colectomy for their gastrointestinal disease. When extensive HS affects the perineal region, as in the first case, it may be difficult to distinguish between HS and Crohn's disease either by clinical or histological criteria. The presence of HS may therefore go unrecognized. However, additional involvement of the axilla favours a diagnosis of HS as metastatic Crohn's disease of the skin usually affects the lower extremities' and has not been reported at this site. The role of local factors in HS has been emphasized'' although the association with Crohn's disease suggests that HS may be a manifestation of underlying disease. Further studies are needed to test the specificity of this association. IX-parlment of Dermatology. Hospital.
N.P.BURROWS R.RtissELL JONES
Road. Soulhall. Middlesex UBl WW. U.K. References 1 Ostlere 1^, Langtry (AA. Mortimer PS, Statjghtoii Rl'll. Hidradenitis suppurativa in Crohn's disease. Or / Dermatol 199 1; 125: i84-f). 2 Kllis Bl, Shier CK, I^isen ]] et ai Acne-associated spondylarthrnpathy: radiographic features. Radiototty 1987; 162: '541-5. i Shum D1\ tJLienlliifr L. Mctastalic Crohn's disease. Arch Dermatol I'^yO; 126: f>45-8. 4 Dvorak VC. Koot RK. Macdregor RR. Host-defence mechanisms in hidradenitis suppurativa. Arch Dermatol 1977: 111: 450-J.
Norwegian scfibies and herpes simplex in a patient with chronic lymphatic leukaemia and hypogammaglobulinaemia SiK. Norwegian scabies is a crusted non-itchy rash caused hy massive infestation with the mite Sarcoptes .^cabiei. This was originally described in patients with Down's syndrome' and leprosy- and more recently in patients who are immunosuppressed by leukaemia. * drugs^ or HIV infection.'^ We report a case of focal crusted scabies occurring on the face of a patient with chronic lymphocytic leukaemia (CLJJ and concurrent herpes simplex. A 67-year-oId man with a lO-year history of CU.. treated with intermittent chlorambuci! and prednisolone, presented with a crusted lesion on his upper lip at the site of a recent herpes simplex infection. He had suffered from recurrent facial herpes for J years and had had an attack of itchy scabies 2 years previously. At the time of presentation he was taking oral acylovir and prednisolone each day. He had been receiving regular infusions of gammagiobulin as he suffered from recurrent chest infections and was hypogammaglobulinaemic. S23
524
CORRESPONDENCE
British Journal of Dermatohgy {1992) 126.
References 1 Wilkinson DS. Norwegian (crusted) scabies, lir I Dermatol 31. 2 Atiderson NP. Stout M. Norwegian scabies. Arch Dcrtmttol Sf n4(): 42: 499-=i()(). i Suzumiya ]. Sumiyoshi .\. Kuroiti V. inmic S. Crusted (Norwegian) scabies wiih adult T-cell leukaemia. Arch Dermatol I98S: ! 2 l : 90i-4. 4. Faterson Wl). Allen BR. Uevcridgt- CW. Norwegian scabies during immunosupprt'ssive therapy. Br \kd I 1973; 4: i l 1-2. 5 Glover A. Young L. Goltz AW. Norwegian scabies in inquired immunodcRciency syndrome: report of ii cast: rt'.sulting in death from associated sep.sis (letlt-rl. / Am Acad Di'rmalol 1987:16; 39()-9.
Scarring alopecia and psoriasis
l-'igure 1. Ik'iivily trusU'd lt'.-,iuns u\ Ninwt'gidii sciibies iiilestiiliiJii at
(he site ol'recenl herpes simplex iiifcilion.
On examination he had gent'ralized lymphadenopathy and heputosplenoincgaly. The lesion on his face was initially vesicular and typical olherpes simplex. A thick crust developed during the next week, spreading out from the nasolabial Ibid and upper lip (Figure 1), Scrapings from the lesion contained large numbers of scabies mites. The lesion resolved completely after 2 months of topical treatment with gamma benzene hexachloride. Norwegian scabies normally affects the hands, feet and trunk and produces a widespread rash suggestive of psoriasis or contact dermatitis. This patient was unusual in that only the face was aftected and that the infestation was so localized. This is the first description of scabies occurring at the same site and at the same time as reactivation of herpes simplex. This case illustrates that impairment of host of defence allows not only infection with atypical organisms but also atypical presentations of more familiar infections. It is important that scabies infestation is included in the differential diagnosis of any immunocompromized patient with a crusted rash, even if other infections are diagnosed at the same site. St Richard's tlospilal Cbichester, W. Sussex. U.K.
C.J.TlBBS D.J.WlLCOX
SIR. Until recently it was believed that hair loss was not a symptom of scalp psoriasis.' It is now well established that psoriatic alopecia is not restricted to patients with erythrodermic and generalized pustular psoriasis, but may al.so occur in patients with chronic plaque p.soriasis.-^ Recently we have shown that hair plucks taken from psoriatic plaques on the scalp have a significantly higher telogen count than normal scalp.' Scarring alopecia in psoriasis has been documented infrequently,^ ^ and we report a further case of this association. The clinical picture and histopathological appearances suggested a single disease entity rather than a coincidental occurrence of psoriasis and scarring alopecia of unknown cause. An 18-year-old woman, suffering from Turners syndrome and epilepsy, presented with a fi-year history of hair loss. She gave no previous history of psoriasis, and there was no family history of psoriasis. She was receiving regular treatment with carbamazepine 200 mg twice daily, and a coinbination ofO-1 5 mg desogestroi and 0 0 2 mg ethinyleslradiol. Her scalp had been treated with topical coal tar and corticosteroid preparations. Examination revealed a large, well demarcated area of scarring alopecia over the occipital and retro-auricular regions of the scalp. Within this area there were no tufts of hair or pustules. Al the margins of the area there was silvery scaling of the scalp which surrounded hair shafts, producing the clinical picture of tinea amiantacea. Skin swabs taken from the border of the area grew Staphtikuocctis utireus; cultures for fungi were negative. Histology of biopsies taken from the scaly margin revealed abnormalities al various levels (Fig. la, and h). The interfollicular epidermis showed hyperkeratosis with orthokeratotic and parakeratotic areas, acanthosis with thinning of the suprapapillary plate, and deep rete ridges. Micn)abscesses of Munro and micropustulesof Kogoj complelett Ihc epidermal picture. The dermis ctintained a mixed intiammatory inliltrate of mononuclear cells and polymorphonuclear leucocytes. The number of hair follicles was decreased. Some damaged hair follicles were observed, partly surrounded and penetrated by leucocytes. No hydropic degeneration was ohserved. The patient was treated with 10% salicylic acid ointment, desoxymethasone cream and betamethasone 1 7-vaIerate scalp application. This treatment resulted in a reduction of the scaling.
