Familial Steatocystoma Multiplex Generalisita Suppuritiva: Oral Rifampicin and Clindamycin Combination Worth a Trial.

Sir,

Steatocystoma multiplex suppurativa (SMS) is an inflammatory variant of steatocystoma multiplex (SM). It is a nevoid condition of the sebaceous glands and may easily be misdiagnosed as hydradenitis suppurativa (HS) unless carefully examined. Both SM and HS present with dermal cysts. In HS, the cysts are usually localized to the flexures, whereas in SM, they tend to be more diffused. Further, the contents of the cysts differ in both conditions. SM contains an oily fluid when punctured, whereas HS has pus. The final diagnosis is a histological one. As the condition is rare, there is a lack of a well-proven, evidence-based treatment modality. We describe here such a rare case report and treatment dilemma, which was later successfully managed with oral rifampicin.

A 19-years-old male presented with a history of multiple asymptomatic skin lesions all over the body for the past ten years. Lately, he was getting intermittent episodes of painful pus discharge from these skin lesions that healed with a scar. On examination, numerous, discrete, mobile, yellowish to skin-colored dermal papules and nodules with cystic consistency of varying sizes, most of which were nontender, were noted all over the body, predominantly over the trunk [Figure 1a], including axilla [Figure 2a], groin, natal cleft, and retro-auricular [Figure 2b] region. Few inflamed lesions with pus discharge were also present over the chest and axilla along with few healed scars [Figure 1a]. The apparently healthy skin also showed similar lesions when pinched lightly [Figure 2c]. On puncturing one of these nodular lesions, extrusion of oily fluid [Figure 1b] was noted. Pus culture from the cysts showed skin commensals. Similar complaints were present in his father and sister [Figure 3]. Histopathological examination of the lesions revealed a dermal cyst lined by stratified squamous epithelium that keratinized without a granular layer and adjacent well-formed sebaceous lobules [Figure 4]. The patient was put on oral isotretinoin (0.5 mg/kg), but he continued to get episodes of inflammation and suppuration even after a month. Subsequently, we added oral rifampicin (600 mg/day) and clindamycin (600 mg/day) as this is considered efficacious in the treatment of HS. The episodes of the suppurative breakdown of the cysts decreased within a month. The patient was doing well until six months in the follow-up.

Figure 1

(a) shows numerous, discrete, mobile, yellowish to skin-colored dermal papules and nodules with few inflamed lesions and few scars; (b) shows extrusion of oily fluid on puncturing one of the nodular lesions

Figure 2

(a) shows hidradenitis-like lesions in the axilla; (b) shows dermal cysts in the retro-auricular area; (c) showing similar lesions when the apparently uninvolved skin was pinched lightly

Figure 3

Dermal cysts with a few healed scars in his sister over the neck (a) and axilla (b)

Figure 4

(a) shows dermal cyst lined by stratified squamous epithelium that keratinized without a granular layer (H and E, 10×); (b) shows well-formed sebaceous lobules adjacent to the cyst wall (H and E, 40×)

SM can be autosomal dominant or sporadic. This report supports the autosomal dominant inheritance, but genetic workup for our patients was not feasible because of cost constraints. The patient presented with HS-like lesions in the axilla, but the clinical findings of dermal cysts were not limited to the flexures and were present diffusely all over the body; the oily fluid on puncturing the dermal cysts, and the characteristic histopathological findings helped us to differentiate this condition from HS. Nevertheless, SMS lesions are found predominantly in the seborrheic areas, with facial and scalp lesions being rare. However, our patient had nodules distributed diffusely all over the body, including the chest, back, face, scalp retro-auricular region, and natal cleft. Thus, it was diagnosed as SM generalasita suppurativa. A similar case has been reported by Lima-Santana CNL et al.[1] and Fekete GL et al.[2].

The treatment of SM has been described mainly via a few case reports and is mostly disappointing. SMS being even rarer, the literature on its treatment modalities is limited [Table 1]. Oral isotretinoin[1345] is of some benefit in stabilizing the condition. Surgical excision and ablative lasers seem to be the only definitive treatment. Incision and drainage,[67] cryotherapy,[28] and tetracycline[9] have also been tried. In our case, the patient became concerned after the dermal cysts started to suppurate and became painful. Oral isotretinoin alone was not enough to control these episodes. Thus it was decided to manage it similar to hidradenitis suppurativa. A recent report shows an improvement of both SMS and HS that were coexisting in a patient by adalimumab,[10] indicating that the pathogenesis of these two conditions might be similar. The case was also having overlapping clinical features, and histopathology helped in making the final diagnosis. HS being more common has better evidence for its management. Based on these ideas, oral rifampicin and clindamycin were added. However, oral isotretinoin was continued for benefit of the doubt. The combination worked well for the patient by decreasing the episodes of painful suppuration and scarring, which was the primary concern of the patient.

Table 1

Tabulation of different treatment modalities used for hidradenitis suppurativa

Authors[Reference], Year	Treatment
Egbert BM et al.,[6] 1979	Incision and drainage with electrocautery
Statham BN et al.,[3] 1984	Isotretinoin
Shwartz JL et al.,[4] 1984	Isotretinoin
Rosen BL et al.,[5] 1986	Isotretinoin
Apaydin R et al.,[8] 2000	Cryotherapy and isotretinoin
Fekete GL et al.,[2] 2010	Cryotherapy and isotretinoin
Gordon Spratt et al.,[7] 2013	Incision & drainage followed bytopical clindamycin solution and benzoyl peroxide wash
Lima Santana CNL et al.,[1] 2016	Isotretinoin
Adams B et al.,[9] 2018	Tetracycline
Atzori L et al.,[10] 2019	Adalimumab

To the best of our knowledge, this modality of treatment for SMS has not been mentioned in the literature, and we believe that oral rifampicin and clindamycin combination can be worth a trial in cases of painful suppurative lesions of steatocystoma multiplex along with the well-described oral isotretinoin. Further, our case cannot answer whether this combination would have worked without the isotretinoin. A trial would be required for the answer.

Declaration of patient consent

Declaration: Consent for publishing the images was obtained from the patient as family members.

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Conflicts of interest

There are no conflicts of interest.