The clinical course of Behçet's disease in pregnancy: a retrospective analysis and review of the literature

Although Behçet's disease is mainly diagnosed during the reproductive periods of life, we know little about the influence of pregnancy on the clinical course of Behçet's disease. Therefore, we analyzed the relationship between Behçet's disease and pregnancy retrospectively, in order to detect any possible interaction between the two multisystemic processes, particularly in regard to the influence of pregnancy on the clinical course of Behçet's disease. We studied 44 pregnancies in 28 women with Behçet's disease. The diagnoses were made according to the criteria of the International Study Group for Behçet's disease. The patients were observed during pregnancy and puerperium periods at monthly intervals. The existence and incidence of symptoms were recorded during these periods. There was remission of Behçet's disease during 23 (52.3%) pregnancies, although the disease had been in a stage of exacerbation before pregnancy. The disease became exacerbated during 12 (27.3%) pregnancies, although it had been in a stage of remission before pregnancy. There were no changes in the clinical course of Behçet's disease in 9 (20.4%) pregnancies. The most frequent manifestations of the clinical exacerbation were increases in the intensity and severity of outbreaks of oral ulcers during pregnancy. Outbreaks of genital ulcers, eye inflammations, and arthritis were other signs of exacerbation. Other than spontaneous abortion in three patients, we did not observe maternal or fetal complications. Although Behçet's disease tends toward remission during pregnancy, the influence of pregnancy on its clinical course is quite variable between patients and even during different pregnancies in the same patient. On the basis of our and previous results, we speculate that pregnancy in general does not seem to markedly affect the natural course of Behçet's disease.     

Herpes gestationis in blacks

To our knowledge, only two cases of herpes gestationis (HG) have been previously reported in black patients. We describe herein two black women with confirmed HG. Both had typical historical and clinical features of the disease. Direct immunofluorescence microscopy demonstrated complement deposition along the dermoepidermal junction in both women. Where HLA typing was possible (in one patient), the combination of HLA-DR3 and HLA-DR4 was found. In addition, anti-HLA-DR2 antibodies were present in serum samples taken from this patient. The occurrence of the HLA-DR3-DR4 combination has been reported to be greatly increased in whites with HG (43%, as compared with a 3% incidence in control subjects). The HLA-DR4 antigen is uncommon in American blacks, which may explain the infrequent occurrence of HG in this population. The occurrence of the DR2 antigen is increased in the husbands of women with HG, and this increase is most pronounced in the husbands of patients with the DR3-DR4 combination. The occurrence of positive immunofluorescence microscopy findings, together with the presence of a rare histocompatibility antigen combination previously associated with HG and the presence of anti-DR2 antibodies in the serum of one of our patients all suggest that HG is pathogenically identical in both blacks and whites.     

Pregnancy and obstetrical outcomes in pseudoxanthoma elasticum

BACKGROUND: Pseudoxanthoma elasticum (PXE) is a genetic multisystem disorder characterized by calcified dystrophic elastic fibres in skin, retina and arteries. Much of the earlier literature on pregnancy in PXE contained reports of severe complications, leading some healthcare providers to advise women with PXE against becoming pregnant and some women with PXE to avoid pregnancy. OBJECTIVES: To evaluate the obstetrical outcomes and the incidence of pregnancy complications in women with PXE and to determine if pregnancy is associated with an adverse effect on the course of the disease. METHODS: Women with PXE (n = 407) answered detailed questionnaires regarding reproductive history and pregnancy as well as the course of their disease. The frequency of reported pregnancy outcomes and complications was determined. Severity indices for the major clinical manifestations of PXE were developed and correlated with gravidity of affected women aged 40 years or over. RESULTS: Among the 306 respondents with PXE who had ever been pregnant, there were 795 pregnancies. Of these, 83% ended in live births and 1% in stillbirth. The median birth weight was within the normal range and the incidence of low birth weight for gestation was low. Hypertension occurred in 10% of pregnancies, gastric bleeding and retinal complications in < 1%, and 12% of pregnancies were associated with worsening of skin manifestations. There was no effect of gravidity and clinical severity on cutaneous (P = 0.07), ocular (P = 0.59) or cardiac (P = 0.42) manifestations of PXE in women aged 40+ years, nor did ever having been pregnant adversely affect these clinical severity indices. Of the 101 women who had never been pregnant, 17% made the decision because they were advised against becoming pregnant by a healthcare professional and 11% did not become pregnant because they feared an adverse outcome either in their pregnancy or disease. CONCLUSIONS: PXE is not associated with markedly increased fetal loss or adverse reproductive outcomes. The incidence of gastric bleeding, although probably higher than in the unaffected population, is much lower than previously reported, and retinal complications are uncommon. Although a few pregnancies were associated with worsening of skin manifestations, there was no correlation of either gravidity or ever having been pregnant with ultimate severity of skin, ocular or cardiovascular manifestations. There is no basis for advising women with PXE to avoid becoming pregnant, and most pregnancies in PXE are uncomplicated.     

Periocular involvement in cutaneous leishmaniasis

OBJECTIVE: Although cutaneous leishmaniasis (CL) occurs mostly in the facial area, periocular involvement accounts for 2-5% of the facial lesions. CL lesions localized in the periocular region can easily be confused with various other diseases. The aim of this study was to examine the frequency of periocular involvement in CL in the Cukurova region of Turkey, as well as the clinical characteristics, diagnosis and methods of treatment of this disease. METHODS: Between December 1998 and December 2004, patients who were diagnosed with CL were evaluated prospectively with respect to periocular involvement. RESULTS: From the 2066 patients evaluated with CL, 2622 lesions were identified. In 59 (2.9%) of these patients, a total of 66 (2.5%) lesions were located in the periocular area. Thirty-two (48.5%) of these lesions were of the papular type, 15 (22.7%) the nodulo-ulcerative type, 10 (15.2%) the plaque type, and nine (13.6%) the nodular type. Dacryocystitis was identified in four patients with periocular involvement. Over the follow-up period, no ocular or periocular deformities or complications developed in these patients. CONCLUSION: Patients suspected of CL should be evaluated and treated early in the course of their disease to prevent any permanent ocular or periocular deformities.     

Secondary autoimmune diseases in herpes gestationis (pemphigoid gestationis).

BACKGROUND: Herpes gestationis (HG) is an autoimmune disease of the skin that occurs exclusively in association with pregnancy (or trophoblastic disease). It is associated with the HLA-DR3 and -DR4 antigens that are also associated with several other autoimmune diseases. HG has previously been reported in association with Graves' disease. OBJECTIVE: Our purpose was to determine the frequency of other autoimmune disease(s) in patients with a history of HG. METHODS: Seventy-five patients with a history of HG were studied for the frequency of other autoimmune diseases. RESULTS: We found an increased frequency of Graves' disease in patients with a history of HG. Those with HG have an increased risk for the development of other autoantibodies. There is an increased frequency of autoimmune diseases in the family members of patients with HG. CONCLUSION: Secondary autoimmune disease in HG is unusual, but does occur. The most frequent is Graves' disease.     

Subungual Squamous Cell Carcinoma: A Case Series

Subungual tumors are rare in general. Of all tumors, subungual squamous cell carcinoma (SSCC) is the most frequent one. Protean clinical presentations and the lack of awareness of the disease are responsible for an incorrect or delayed diagnosis and subsequent delayed treatment. We have reported here four patients with SSCC who were previously wrongly diagnosed with a benign process and treated unsuccessfully for years. We would like to highlight the need of a biopsy in chronic or recurrent nail lesions that fail to respond to a previous conservative treatment in order to rule out SSCC.     

Safety of pregnancy after discontinuation of isotretinoin

Isotretinoin (13-cis-retinoic acid, Accutane) increases the risk of major congenital malformations in infants exposed to isotretinoin during pregnancy. However, there have been no epidemiologic reports to date on the effect of a subsequent pregnancy after discontinuation of isotretinoin. This article describes our analysis of pregnancy case reports from patients in whom conception occurred after isotretinoin treatment had been discontinued. Based on the 88 prospectively ascertained cases, the incidence rate of both spontaneous and missed abortions from all pregnancies was 9.1% (eight patients), and the incidence rate of congenital malformation among the live births was 5.0% (four patients). The incidence rates for both these outcomes were not significantly different from the rates reported for women of reproductive age in the general population. In addition, the malformations reported were not characteristic of retinoic acid-induced congenital anomalies.     

Characteristic cutaneous lesions in patients with brucellosis

Among 436 cases of brucellosis included in a 12-year prospective protocol, we identified 27 patients (6%) with cutaneous lesions (13 men and 14 women; mean age, 35.1 +/- 16.5 years). Twenty-one patients had positive blood cultures for Brucella melitensis. A disseminated violet-erythematous, papulonodular eruption (20 cases) and erythema nodosum-like lesions (three cases) were the most frequent eruptions observed, appearing during the initial episode of the disease or in relapse. Histologic findings were a dermal inflammatory infiltrate of lymphocytes and histiocytes in a perivascular and periadnexal arrangement, with a focally granulomatous appearance, and occasional extension to subcutaneous fat. Skin cultures were positive for B melitensis in two of four cases. Our results suggest that there are characteristic clinical and histologic cutaneous findings in patients with brucellosis and that hematogenous spread of the microorganism can be the most important pathogenic mechanism of these lesions.     

Heavy multinodular cutaneous lymphoid infiltrates: clinicopathologic features and B-cell clonality

BACKGROUND: Criteria for distinguishing between cutaneous lymphoid hyperplasia (CLH) and low-grade B-cell lymphoma are not well defined. We examined the hypothesis that the presence of a clonal B-cell population in heavy multinodular lymphoid infiltrates correlates with clinical presentation and outcome. METHODS: We identified 29 patients with skin lesions characterized histologically by a heavy dermal lymphocytic infiltrate with a multinodular architecture and extension into deep dermis and subcutaneous fat. Clonality was assessed immunophenotypically by light-chain restriction and also by analysis for IgH-gene rearrangement using PCR on DNA extracted from paraffin blocks. RESULTS: Follow-up (mean 80 months; median 45 months) was obtained in all patients. Twenty-four patients (83%) presented with a solitary lesion: only four had solitary recurrences, and none developed multiple synchronous lesions or systemic B-cell lymphoma. However, 9/24 of these solitary lesions (38%) were clonal by light-chain restriction or IgH PCR; 5/29 patients (17%) presented with multiple recurrent lesions and continued to develop lesions during the period of follow-up; 3/5 patients (60%) with multiple lesions demonstrated a B-cell clone. No patient developed systemic B-cell lymphoma. CONCLUSIONS: Heavy, multinodular cutaneous lymphoid infiltrates have an excellent prognosis. Multiple lesions at presentation are the best predictor of recurrent multiple lesions confined to the skin. The presence of a clonal B-cell population does not correlate with clinical presentation or histology, nor does it predict development of further lesions or systemic lymphoma.     

Increased psychiatric morbidity in female outpatients with skin lesions on visible parts of the body

Psychiatric disorders are frequent among patients with skin diseases. We aimed at identifying factors associated with psychiatric morbidity in dermatological outpatients. All adults attending the outpatient clinics of a dermatological hospital on predetermined days were given the 12-item General Health Questionnaire. The dermatologists indicated the diagnosis and location of skin lesions and rated the disease severity. A total of 1389 patients were asked to take part in the study. Of the 722 who accepted, 389 had a complete set of data and were included in the analysis. To verify the representativeness of our sample, we used the administrative registries to compare participants with the total population of patients who attended the clinics during the same period, and we examined the distribution of missing data. There was a tendency towards a younger age in the sample studied, but no evidence of substantial selection bias. The prevalence of psychiatric morbidity was 20.6%. We found higher probability of psychiatric disorders in women, controlling for age, clinical severity and localization of lesions. In women, but not in men, the prevalence of psychiatric morbidity was higher in patients with lesions on the face or hands. Given that the identification and appropriate management of psychiatric morbidity are important, it seems that the dermatologist should be particularly alert to the possibility of a concurrent psychiatric disorder in women with lesions on the face or hands.     

Pimecrolimus 1% cream in the treatment of vulvar lichen sclerosus in postmenopausal women

Background Vulvar lichen sclerosus (LS), a poorly recognized chronic inflammatory skin disease, may represent a therapeutic challenge. Pimecrolimus cream 1% is a nonsteroidal, selective inflammatory cytokine inhibitor that has recently been indicated for some inflammatory cutaneous diseases. Objective To investigate the efficacy, tolerability, and safety of 1% pimecrolimus cream therapy in postmenopausal women with LS. Methods A total of 16 patients applied pimecrolimus cream 1% twice a day over the first 3 months and then as required. All the patients completed this study and were then followed up over the next 12 months. The symptoms and clinical appearance of the lesions in every subject were recorded before and after treatment using a severity scale. Results Using pimecrolimus, most of the patients exhibited a significant improvement with respect to symptoms and the clinical appearance of the disease. After 3 months of treatment, complete remission was seen in 11 patients, partial remission in four, whereas one patient experienced no response at all. Over the subsequent 12 months of follow‐up, 10 patients exhibited complete remission while five had partial remission. Four cases with complete remission experienced a few relapses during the follow‐up period. Older patients and those with an advanced stage of the disease responded poorly. No significant side effects were observed. Conclusions Pimecrolimus cream 1% appears to be an effective and well‐tolerated therapeutic alternative option in the treatment of early stage of vulvar LS. Pimecrolimus may reduce the incidence of flare ups, improve long‐term disease control, and enhance the patients’ quality of life, especially in postmenopausal women.     

Urticaria in Infants: A Study of Forty Patients

A retrospective study was carried out in 40 infants, age 1 to 24 months, with urticaria. Acute urticaria was seen most frequently (85%), followed by recurrent (10%) and chronic disease (5%). Several clinical features such as frequency of angioedema and hemorrhagic lesions appeared to be specific to urticaria in infants. An underlying cause was identified or suspected in 65% of cases: foods in 25%, and drugs and infections in 37.5%. Under 6 months of age, all infants had acute urticaria and 75% had cow's milk allergy. After 6 months of age, the main causes were drug intake (mostly aspirin and amoxicillin) and/or infections (mainly viral) (50%). Atopy was not overrepresented (20%), although a possible link between atopy and recurrent urticaria was noted. After a follow-up of 2 to 7.5 years, 96% of patients were symptom free. Thus, our results indicate that clinical and etiologic features of urticaria in infants are somewhat different from those of adults and children.     

BEHÇET'S DISEASE: CLINICAL STUDY OF JORDANIAN PATIENTS

Background. Behçet's disease is a chronic, recurrent, inflammatory disorder characterized by the triad of oral and genital ulcers and ocular lesions. The etiology is unknown. This is the first report giving details of the clinical manifestations of Behçet's disease in Jordanian patients. Methods. Twenty patients with Behçet's disease were studied to determine the clinical pattern in the North of Jordan. The patients were seen in Princess Basma Teaching Hospital in North Jordan. They presented to various clinics and underwent full clinical examination. Data for each patient on all features of Behçet's disease were recorded on a standard form. A comparison was made between Behçet's disease in Jordan and other countries in the region. Results. Of the 20 patients, 14 were men and six women, giving a ratio of 2.3:1. Their ages ranged from 14 to 58 years. All had mouth ulcers, 65% genital ulcers, 65% ocular involvement, 55% joint involvement, 35% skin lesions, 20% vascular lesions, and 5% gastrointestinal involvement. The oral ulcers were the first manifestation of the disease process in 70% of our patients. Skin lesions, genital ulcers, and involvement of the central nervous system and the pulmonary system were less frequent in our study. Conclusions. Although it is difficult to obtain figures as to the incidence and prevalence of Behçet's disease in Jordan, the clinical manifestations, with a few exceptions, are similar to those in other countries in the region.     

Kaposi's sarcoma in women: A clinicopathologic study

BACKGROUND: Although all epidemiologic subsets of Kaposi's sarcoma (KS) (i.e., sporadic, endemic, epidemic, and iatrogenic) have an association with human herpes virus 8 (HHV8), these subsets occur in patient populations with distinctive clinical features. To a variable degree men outnumber women in all subsets. OBJECTIVE: A retrospective study of women with histologically proven cutaneous KS was undertaken to determine the clinical and histopathologic features, as well as any associations. METHODS: Two hundred and fifty cases of cutaneous KS in women from 1975 to 1993 were reviewed. RESULTS: Of the patients, 80% were more than 60 years of age, and of the patients less than 60 years old, 28 were from areas of the world with endemic KS. All HIV+ patients but one were from areas of endemic KS. Two patients were renal transplant patients. Sixty-four percent of the patients had single lesions and 21% recurrent lesions. Twelve patients had, or were known to develop, internal involvement, and in six patients the cause of death was KS. All but four cases histologically showed areas of solid proliferations of tumour cells consistent with plaque or tumour stage. An angiosarcoma-like histologic pattern appeared to be associated with more aggressive epidemiologic subsets. High mitotic rates were rarely seen and did not correlate with aggressive epidemiologic subsets. CONCLUSION: Kaposi's sarcoma in women is diagnosed almost exclusively in plaque or tumour stage. The majority of women within our study fit within the epidemiologic subset of sporadic KS.     

Early-stage mycosis fungoides, parapsoriasis en plaque, and pregnancy

BACKGROUND: Non-Hodgkin's lymphoma (NHL) coincident with pregnancy is rare, and the literature regarding mycosis fungoides (MF), the most common primary cutaneous NHL, and pregnancy is strikingly sparse. The effect of pregnancy on MF, or on parapsoriasis en plaque (PPP), and the effect of these diseases on pregnancy, are still unknown. OBJECTIVE: To study the effect of pregnancy on MF and PPP and the impact of these diseases on pregnancy. METHODS: The files of the MF and PPP patients seen during the past 12 years in our department were reviewed to search for patients who had been pregnant during the course of their disease. RESULTS: Nine women who met the study criteria were identified, seven with early-stage MF and two with PPP. A total of 12 pregnancies was recorded: nine in patients with MF and three in patients with PPP. In none of the patients was there any indication that pregnancy changed the course of MF or PPP. Of the 12 pregnancies, 11 were normal; one was naturally aborted. Two of the patients were treated with topical steroids during pregnancy. One patient was treated with narrow-band ultraviolet-B combined with topical steroids. The rest preferred to avoid any therapy. CONCLUSIONS: Pregnancy appeared to have no impact on the course of early MF or PPP, and no adverse effect was noted on pregnancy. Further studies are needed to clarify the interplay between pregnancy and MF or PPP.     

The spectrum of mucocutaneous manifestations in Adamantiades‐Behçet’s disease in Greece

Background This is the largest specific demographic and clinical study performed until now in Greece. Objectives To analyse the spectrum of mucocutaneous manifestations in 202 patients with Adamantiades‐Behçet’s disease (ABD) in Greece. Methods Any mucocutaneous symptom at disease onset and during the follow‐up was recorded in a particular questionnaire that included 58 items. All patients fulfilled the International Study Group Criteria for BD. Results Consecutive patients (130 men and 72 women) were included in this study. Their mean age was 42.03 ± 12.41 and 44.96 ± 11.99 years for male and female patients respectively. Pathergy test was positive in 38%, whereas HLA‐B5 (51) positivity was evident in 76% of patients. Onset signs: oral aphthous ulcers were found in 64.36%, genital ulcers in 6.93%, skin lesions in 8.91%, erythema nodosum in 7.42% and pseudofolliculitis in 1.5%. One patient had leg ulcers. During the follow‐up, oral aphthous ulcers were found in 100%, genital ulcers in 65.4% and in 51.4%, erythema nodosum in 42.9% and in 78.1%, whereas pseudofolliculitis in 57.1% and in 21.9% in men and women respectively. Significant differences pointing to a different course of mucocutaneous disease were found between men and women. Conclusions The nature and frequency of mucocutaneous manifestations at presentation are important for the diagnosis of ABD, whereas significant differences were found between genders in this Greek patient cohort. Significant differences were also observed when our results were compared with those of four other series, probably explained by genetic and environmental factors.     

Recurrent aphthous stomatitis: treatment with colchicine. An open trial of 54 cases

INTRODUCTION: Colchicine is a treatment used for aphthous stomatitis but its efficacy has never been proved. We report the results of an open trial of 54 patients treated with colchicine for aphthous stomatitis. The aim of our study was to assess the long and short term efficacy and tolerance of this molecule in this disease. PATIENTS AND METHODS: The case reports of 64 patients presenting with severe, recurrent aphthous stomatitis treated with colchicine between 1986 and 2000 were analyzed retrospectively. Only immunocompetent patients exhibiting idiopathic aphthous stomatitis or Beh?et's disease were included. Ten patients were excluded: 4 cases of Beh?et's disease treated with colchicine and systemic corticosteroids, 4 cases of aphthous stomatitis secondary to a hematological cause and 2 patients who were lost to follow-up after 3 month's treatment. Colchicine was prescribed at a dose of 1 to 1.5 mg/d for at least 3 months. All the patients were assessed after 3 month's treatment and all were contacted by phone in May 2000. Our end-point criteria were: the frequency and duration of the episodes, intensity of pain and impact on the quality of life of the patients. RESULTS: Fifty-four patients were included: 39 women (mean age=44 +/- 16.8) and 15 men (mean age=49 +/- 13.5). The disease had progressed for a mean of 11.6 +/- 13.5 years. After 3 month's treatment, 12 patients (22 p. 100) no longer had aphta and were in complete remission, 22 patients (41 p. 100) were significantly improved, since the frequency and duration of the lesions had decreased by at least 50 p. 100 and the latter were no longer painful. Treatment failed or tolerance was poor in 20 patients (37 p. 100). Patients were monitored and assessed for a mean of 4.7 years (range: 6 months to 13 years). Six patients were lost to follow-up. Three patients (6 p. 100) were considered as cured, since they were still in complete remission after suspension of colchicine respectively 6, 24 and 72 months later. Fifteen patients (31 p. 100) had still improved. Among the latter, 10 continued colchicine for a mean of 27 months, and 5 patients stopped treatment, which they considered tedious, after a mean of 16 months. These 15 patients noted a significant reduction in the frequency and duration of the lesions. Moreover, the subjective end-point criteria had improved significantly for them not to wish to change treatment. Thirty patients (63 p. 100) were not improved by the treatment. Some benign adverse events were noted in 10 patients (18.5 p. 100) and led to suspension of treatment in 4 cases. DISCUSSION: This study, which is the first in size (n=54) and duration of follow-up (4.7 years), showed that colchicine is an efficient preventive treatment of severe aphthous stomatitis for 63 p. 100 of patients after 3 month's treatment and that this improvement was lasting in 37 p. 100 of them. Colchicine should therefore be proposed in first intention in severe recurrent aphthous stomatitis, since it is effective, well tolerated and easy to use.     

Treatment of chromoblastomycosis with itraconazole, cryosurgery, and a combination of both

Background Chromoblastomycosis is a subcutaneous mycosis, seen frequently in tropical areas, and caused by dematiaceous fungi. It produces nodulo-verrucous lesions in the arms and legs. There is no treatment of choice for this disease and sometimes a combination of chemotherapy and physical therapy is necessary. Methods The study included 12 patients diagnosed with chromoblastomycosis by means of fungal and histopathologic tests. The patients were assigned to three treatment groups: patients with small lesions, not greater than 15 cm² in area, were assigned to Group 1, in which the treatment consisted of itraconazole 300 mg/day, or to Group 2, in which the treatment consisted of one or more sessions of open-spray cryosurgery. Patients with large lesions were assigned to Group 3 and started treatment with itraconazole 300 mg/day, until a maximal reduction of lesions occurred, and then underwent one or several cryosurgery sessions. Clinical, fungal, and laboratory tests were performed in each group before, during, and at the completion of treatment. Results Positive cultures of Fonsecaea pedrosoi were obtained in 11 out of 12 patients. Two out of four patients in Groups 1 and 3 had a clinical and fungal cure and the remaining patients experienced significant improvement. All four patients included in Group 2 achieved a cure. No important side-effects were seen among the patients included in any of the two itraconazole groups, and only two out of eight patients reported gastric discomfort. The cryosurgery group reported only normal complications of the process, such as edema and pain; two out of eight patients had a superimposed infection. Conclusions The results of itraconazole and cryosurgery were good in cases with small lesions; antifungal therapy being more appropriate for flexion areas. The combination of itraconazole, to reduce the size of the lesions, with subsequent treatment of the remaining lesions with cryosurgery, represents a new alternative in the treatment of patients with large lesions. Both types of therapy are considered safe, with few side-effects.     

Perianal hidradenitis suppurativa: early surgical treatment gives good results in chronic or recurrent cases

Hidradenitis suppurativa (HS) represents a chronic, recurrent, deep-seated folliculitis resulting in abscesses followed by the formation of sinus tracts and scarring. It is an uncommon disorder frequently involving the perianal region. We review the clinical and pathological features of 12 patients (10 men and two women) with perianal HS (PHS). The mean age at presentation was 43 years (range 24–65), and the mean duration of illness was 14 years (range 2–30). Two patients exhibited multiple areas of involvement, including the axilla and the nape of the neck, in addition to the perianal region. Histological examinations showed epidermis-lined sinuses surrounded by dense plasma cell infiltration in four of nine specimens. The complications related with PHS included anal fistula (seven patients), diabetes mellitus (four), malignant tumour (two) and sepsis (one). Three patients died of the complications. Eight patients underwent wide local excision. The defects were managed with a meshed split-thickness skin graft in five patients, and open treatment in the remainder. New lesions developed in untreated skin of two patients. However, no patient relapsed in the surgically treated sites. PHS is frequently complicated with anal fistula and is occasionally life-threatening as a result of the occurrence of malignant tumours or sepsis. Early surgical excision followed by meshed split-thickness skin graft can successfully cure the lesion without functional disturbance.