Perforating dermatoses L87.1

The diseases belonging to the group of perforating dermatoses were described by the following persons:

• Kyrle's disease(hyperkeratosis follicularis et parafollicularis in cutem penetrans) in 1916
• Elastosis perforans serpiginosa in 1953 by Lutz
• Perforating folliculitis
• Acquired reactive perforating collagenosis in 1968 by Mehregan and Coskey.

This group of diseases is characterized by the phenomenon of transepidermal elimination of denatured collagenous connective tissue from the dermis as a histopathological finding. In 1989, Rapini et al. summarized it as acquired perforating dermatosis. However, as there are also cases of early-onset disease that are not necessarily acquired, they are currently often referred to collectively as perforating dermatosis. The pathological finding of transepidermal elimination, in which denatured skin components are excreted outside the skin, is essential as a diagnostic criterion for perforating dermatosis.

Perforating dermatoses are a heterogeneous group of diseases characterized by a transepidermal expulsion of collagen and/or elastin through the skin (Schmults CA 2002). As prototypes of a perforating dermatosis the "Elastosis perforans serpiginosa", the "Reactive perforating collagenosis" are considered (see overview).

• Reactive perforating collagenosis
• Perforating folliculitis (Folliculitis perforans)
• elastosis perforans serpiginosa
• Kyrle's disease
• Perforating granuloma anulare
• Perforating pseudoxanthoma
• Acquired perforating dermatoses in the context of underlying diseases such as diabetes mellitus and chronic terminal renal failure.

Aetiopathogenetically, perforating skin diseases can be divided into 2 categories (Lukasz J et al. 2018):

• Inherited perforating dermatoses
• Acquired perforating dermatoses

The treatment of perforating dermatoses is not uniformly standardized. There are no controlled studies and no treatment guidelines. To be mentioned:

• Topical intralesional corticosteroids
• Topical retinoids
• Topical vitamin D analogues (e.g. Maxacalcitol)
• Topical cantharidine (Wong J et al. 2012)
• Systemic Retinoids: Acitretin mg/day (Satchell AC et al. 2001)
• Systemic antihistamines possibly in combination with UVB irradiation
• Allopurinol (100 mg/day): Smaller studies exist (Lukacs J et al. 2018). Possibly in combination with antibiotics and PUVA therapy.
• Systemic antibiotics: Successes have been achieved with clindamycin, flucloxacillin, levofloxacin, doxycycline, roxithromycin and minocycline (see below for an overview of these drugs, Lukacs J et al. 2018).
• Dapsone (50mg/day): A case report exists with a positive result (Tsuboi H et al. 2004)
• Amitryptyline (initial 10mg- as maintenance therapy 25mg/day): Treatment protocols for 2 patients are available (Yong A et al. (2014)
• Phototherapy (PUVA and NB-UVB): There are numerous case reports on these forms of therapy (see below Lukacs J et al. 2018)

1. Kawakami T et al. (2020) Clinical practice guide for the treatment of perforating dermatosis. J Dermatol 47:1374-1382
2. Lukacs J et al. (2018) Treatment of acquired reactive perforating dermatosis-a systematic review. JDDG 16: 825-844
3. Satchell AC et al (2001) Reactive perforating collagenosis. A condition that may be underdiagnosed. Australas J Dermatol 42: 284-287
4. Schmults CA (2002) Acquired reactive perforating collagenosis. Dermatol online J 8: 8
5. Tsuboi H et al. (2004) Acquired perforating collagenosis in a patient with lung fibrosis. J Dermatol 3:916-919
6. Wong J et al. (2012) Treatment of acquired perforating dermatosis with cantharidin. Arch Dermatol 148: 160-162
7. Yong A et al. (2014) Effective treatment of uremic pruritus and acquired perforating dermatosis with amitryptiline. Australas J Dermatol 55: e54-57